NextGen 2024: Refractory SJIA & MAS Session Part 6

NextGen 2024: Refractory SJIA & MAS Session Part 6

@SJIA_Foundation
@SJIA_Foundation
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2 months ago 183

International perspectives (China): Presentation from Dr. Caifeng Li

NextGen 2024: Refractory SJIA & MAS Session Part 6

@SJIA_Foundation2 months ago

Systemic Juvenile Idiopathic Arthritis in China

Caifeng Li, MD, PhD

Professor of Capital Medical University Chief of Pediatric Rheumatology Department, Beijing Children's Hospital, National Medical Center for Children Chief of Rheumatology Department, Capital Medical University Executive President of Beijing Children's Hospital Zhengzhou Hospital

                        Contents

                        • glyph Treatment of sJIA
                        • glyph Treatment of sJIA in Beijing Children's Hospital
                        • ◆ sJIA with MAS
                        • ◆ sJIA with lung involvement
                        • ◆ refractory sJIA
                        • glyph Unmet needs and future perspectives

                                              Treatment of sJIA

                                              glyph Commonly used medications for sJIA in China:

                                              • ◆ Glucocorticoids
                                              • ◆ NSAIDs
                                              • ◆ Cyclosporine
                                              • ◆ Thalidomide
                                              • ◆ Tocilizumab
                                              • ◆ JAK inhibitors: Tofacitinib
                                              • glyph Treatment Plan
                                              • ◆ general treatment:
                                              • ● GC+ NSAIDs+ Cyclosporine+ Thalidomide
                                              • ● GC+ NSAIDs+ Tocilizumab
                                              • ◆ refractory sJIA treatment:
                                              • ● GC+ NSAIDs+ Cyclosporine+ Thalidomide+ Tocilizumab/ Tofacitinib
                                              • ● TNF inhibitors added for patients with chronic arthritis

                                                                    Treatment of sJIA in BCH

                                                                    glyph Initial treatment data of 546 sJIA cases in BCH

                                                                    • ◆ Glucocorticoid: 545 patients (99.8%) initially received, with 501 (91.8%) receiving one dose of MP pulse therapy, 20 received 2 doses MP pulse, 3 received 3 doses MP pulse
                                                                    • ◆ Most commonly used DMARDs: CSA (96.3%), MTX (8.4%, primarily for arthritis)
                                                                    • ◆ 41 cases (7.5%) used Thalidomide
                                                                    • ◆ Biologics and JAK inhibitors: Tocilizumab was the primary choice (16.8%), and 31 cases (5.7%) received Tofacitinib treatment

                                                                                          Treatment of sJIA in BCH

                                                                                          glyph Follow-up data of 260 sJIA cases in BCH

                                                                                          • ◆ Treatment plan: Glucocorticoids + NSAIDs + DMARDs + biologics/JAK inhibitors
                                                                                          • ◆ At 1 month, 74.2% of patients achieved ACRp30 response
                                                                                          • ◆ At 3 months, 94.2% achieved ACRp30 response
                                                                                          • ◆ By 24 months
                                                                                          • ● 100% of patients reached ACRp30 response
                                                                                          • ● 95.4% achieved ACRp50 response
                                                                                          • ● 75% reached ACRp70 response

                                                                                                                sJIA with MAS in BCH

                                                                                                                • glyph Clinical data of 141 sJIA-MAS cases in BCH
                                                                                                                • glyph 94 males and 47 females, male-to-female ratio 2:1
                                                                                                                • glyph
                                                                                                                • Average age of onset: 7.6±3.4 years
                                                                                                                • glyph 16 (11.3%) cases of onset with MAS
                                                                                                                • glyph Clinical Manifestations: the main clinical features included fever (100%), hematologic involvement (95%), and respiratory involvement (90%)
                                                                                                                • ◆ Respiratory involvement:
                                                                                                                • ● pleural effusion: 38 cases (27%)
                                                                                                                • ● acute respiratory distress syndrome: 11 cases (5.7%)

                                                                                                                                      sJIA with MAS in BCH

                                                                                                                                      glyph Treatment data of 70 sJIA-MAS cases in BCH

                                                                                                                                      • ◆ 70 (100%) received MP pulse therapy
                                                                                                                                      • ◆ 48 (68.6%) received cyclosporine
                                                                                                                                      • ◆ The most common treatment plan:
                                                                                                                                      • ● MP pulse+CSA+ IVIG (38.6%)
                                                                                                                                      • ● followed by MP pulse + IVIG (28.6%)

                                                                                                                                                            sJIA with lung involvement in BCH

                                                                                                                                                            • glyph Data of 280 sJIA cases in BCH, including 38 (13.6%) cases with lung involvement
                                                                                                                                                            • glyph male-to-female ratio of 1.5:1, average age of onset: 5.7 ± 3.0 years
                                                                                                                                                            • glyph Among patients with lung involvement, 13 (34.2%) cases had MAS
                                                                                                                                                            • glyph Types of lung involvement:
                                                                                                                                                            • ◆ the main type was interstitial lung disease (92.1%)
                                                                                                                                                            • ◆ pulmonary hemorrhage (10.5%), pulmonary hypertension (5.3%)
                                                                                                                                                            • glyph Clinical Manifestations: fever and respiratory symptoms
                                                                                                                                                            • ◆ mainly cough and chest tightness, 13 (34.2%) required respiratory support
                                                                                                                                                            NSIP: Non-specific interstitial pneumonia
                                                                                                                                                            OP: Organizing pneumonia SJA
                                                                                                                                                                                  • glyph

                                                                                                                                                                                  sJIA with lung involvement in BCH

                                                                                                                                                                                  Initial treatment: GC + NSAIDs + DMARDs

                                                                                                                                                                                • ◆ 37 (97.4%) used GC, with 73.3% receiving MP pulse therapy
                                                                                                                                                                                • ◆ 17 (44.7%) used cyclosporine
                                                                                                                                                                                • ◆ 6 (15.8%) added Tocilizumab/JAK inhibitors
                                                                                                                                                                                • glyph Follow-up: 32 patients for 5-105 months
                                                                                                                                                                                • ◆ 26 (81.3%) showed improvement
                                                                                                                                                                                • ◆ 22 (68.7%) added tocilizumab
                                                                                                                                                                                • ◆ 5 (15.6%) added tofacitinib
                                                                                                                                                                                • ◆ 1 patient stopped all medications
                                                                                                                                                                                • ◆ 10 cases (31.3%) stopped GC
                                                                                                                                                                                • ◆ 3 (9.4%) died due to MAS, pulmonary hemorrhage, respiratory failure, ARDS, and renal failure

                                                                                                                                                                                                      sJIA with lung involvement in BCH

                                                                                                                                                                                                      glyph HRCT scans of lungs in some patients during follow-up

                                                                                                                                                                                                      Before treatment: sJIA-ILD After treatment (58 months)
                                                                                                                                                                                                      Before treatment: sJIA-ILD After treatment (89 months)

                                                                                                                                                                                                      Before treatment: sJIA-ILD After treatment (22 months) (Organising pneumonia, OP)

                                                                                                                                                                                                      Before treatment: sJIA-ILD+MAS After treatment (5 months)

                                                                                                                                                                                                                            refractory sJIA in BCH

                                                                                                                                                                                                                            • glyph Data of 11 refractory sJIA cases in BCH: 7 males, 4 females
                                                                                                                                                                                                                            • ◆ Reasons for relapse:
                                                                                                                                                                                                                            • ◆ At onset: average age 3.5 years, 5 onset with MAS, 5 had lung involvement
                                                                                                                                                                                                                            • ● MAS (7, 63.6%), 5 patients experienced 2 times of MAS
                                                                                                                                                                                                                            • ● Infection caused disease activity (6, 54.5%)
                                                                                                                                                                                                                            • ● Chronic arthritis (2, 18.2%)
                                                                                                                                                                                                                            • ◆ Outcomes: 2 give up due to multiple organ failure, 3 achieved disease control with tocilizumab, 5 achieve control with Tofacitinib, and 4 (36.4%) stop GC

                                                                                                                                                                                                                                                  Unmet needs and future perspectives

                                                                                                                                                                                                                                                  • glyph Subsequent Plan - sJIA Database Construction in China
                                                                                                                                                                                                                                                  • ◆ Construct a nationwide multicenter sJIA database
                                                                                                                                                                                                                                                  • ◆ Incorporating 34 tertiary medical institutions
                                                                                                                                                                                                                                                  • ◆ Plan to complete the database construction by December 2024
Systemic Juvenile Idiopathic Arthritis 
in China
Caifeng Li, MD, PhD
Professor of Capital Medica…
1/13
 Treatment of sJIA
 Treatment of sJIA in Beijing Children's Hospital
◆ sJIA with MAS
◆ sJIA with…
2/13
 Commonly used medications for sJIA in China:
◆ Glucocorticoids 
◆ NSAIDs
◆ Cyclosporine
◆ Thal…
3/13
 Initial treatment data of 546 sJIA cases in BCH
◆ Glucocorticoid: 545 patients (99.8%) initially …
4/13
 Follow-up data of 260 sJIA cases in BCH
◆ Treatment plan: Glucocorticoids + NSAIDs + DMARDs + bio…
5/13
 Clinical data of 141 sJIA-MAS cases in BCH
 94 males and 47 females, male-to-female ratio 2:1
 A…
6/13
 Treatment data of 70 sJIA-MAS cases in BCH
◆ 70 (100%) received MP pulse therapy
◆ 48 (68.6%) re…
7/13
 Data of 280 sJIA cases in BCH, including 38 (13.6%) cases with lung involvement
 male-to-female r…
8/13
 Initial treatment: GC + NSAIDs + DMARDs
◆ 37 (97.4%) used GC, with 73.3% receiving 
MP pulse the…
9/13
sJIA with lung involvement in BCH
 HRCT scans of lungs in some patients during follow-up 
Before …
10/13
refractory sJIA in BCH
Onset Follow-up Relapse Refractory Reason MAS Treatment Changes Final Follo…
11/13
 Subsequent Plan - sJIA Database Construction in China
◆ Construct a nationwide multicenter sJIA d…
12/13
谢谢各位聆听
THANK YOU!
13
13/13


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